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Introduction: Hepatoid adenocarcinoma (HAC) is a rare neoplasm with a poor prognosis that shows morphological and biochemical similarities to hepatocellular carcinoma. It can occur at any anatomical site, predominantly in the stomach. So far, HAC of soft tissue origin has only been reported once in the literature. Case presentation: We report an unusual case of a 64-year-old male patient diagnosed with a HAC of the soft tissue, with production of alpha fetoprotein. The patient was treated with systemic therapy and responded clinically, radiologically and biochemically to treatment with cisplatin and etoposide. Conclusion: When a patient presents with an extra-hepatic mass, morphologically mimicking hepatocellular carcinoma, the diagnosis of HAC should be considered, especially in case of elevated serum levels of alpha fetoprotein. The disease may be sensitive to systemic treatment.